Langerhans cell histiocytosis (LCH) is a rare neoplastic disorder of abnormal proliferation of Langerhans cells affecting predominantly pediatric populations; sites of involvement include cutaneous, osseous, hematopoietic, and multisystem disease (1). Cutaneous involvement is common in children, though thymic involvement is rarely described (2). We report a case of an 8-month-old female infant with a dyad of cutaneous and thymic LCH. The purpose of this paper is to highlight a potentially underdiagnosed manifestation of infantile LCH and consider thymic sonography in infant LCH staging evaluation. A one-month old female presented with skin lesions and pruritus suspicious for atopic dermatitis failing to respond to therapy. Punch biopsy demonstrated cutaneous LCH. Staging non-contrast chest computed tomography (CT) showed multiple very faint calcifications in the thymus (figure 1) which could have been obscured by intravenous contrast administration. Ultrasound demonstrated multiple echogenic foci in the thymus (figure 2). Pathology confirmed thymic LCH. Throughout her presentation, the patient’s clinical symptoms of LCH were limited to diffuse pruritic cutaneous lesions. Thymic involvement changed patient management to chemotherapy infusion. At the time of this report she has shown no signs of disease progression and she remains clinically stable.
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Meeting name:
SPR 2023 Annual Meeting & Postgraduate Course
, 2023
Authors:
Goldstein Elianna,
White Elena,
Giamanco Nicole,
Rooks Veronica
Keywords:
Langerhans Cell Histiocytosis,
Ultrasound,
Thymus